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The embryonic expression of the gene has not been studied in detail before. Moreover, the protein <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 was mostly localized on the subcellular level to lysosomes but the exclusiveness is still under debate. Here, we analyze the expression pattern of murine <jats:italic><jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3<\/jats:italic> at different developmental stages by <jats:italic>in\u00a0situ<\/jats:italic> hybridizations. We observe expression maxima in the developing thalamus and cerebral cortex and outside of the central nervous system in the gastrointestinal tract and other peripheral organs. In differentiated primary neurons, the protein <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 shows mainly a somatodendritic localization. In primary neurons, we thoroughly revisit the subcellular localization of <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 and find a predominant localization in late endosomal\u2013lysosomal compartments. Moreover, we expressed the major mutant form of <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 \u2013 <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3deltaExon7\/8 \u2013 in neurons and demonstrate that it is retained in the endoplasmatic reticulum. Time\u2010lapse microscopy analysis of neurons revealed co\u2010trafficking of <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 with the late endosomal marker Rab7, but not with the early endosomal marker Rab5. Furthermore, a constitutive active mutant of Rab7 traps <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 in enlarged endosomes. Our subcellular localization study in neurons refines the localization and subcellular targeting of <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 to late endosomal\u2013lysosomal compartments and provides information on the velocity of <jats:styled-content style=\"fixed-case\">CLN<\/jats:styled-content>3 in living neurons which has not been investigated before.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc13744-fig-0008-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text>\n<\/jats:p><\/jats:sec>","DOI":"10.1111\/jnc.13744","type":"journal-article","created":{"date-parts":[[2016,7,25]],"date-time":"2016-07-25T11:37:06Z","timestamp":1469446626000},"page":"456-470","source":"Crossref","is-referenced-by-count":26,"title":["Revisiting the neuronal localization and trafficking of <scp>CLN<\/scp>3 in juvenile neuronal ceroid lipofuscinosis"],"prefix":"10.1111","volume":"139","author":[{"given":"Sandra","family":"Oetjen","sequence":"first","affiliation":[{"name":"Institute for Molecular and Cellular Cognition Center for Molecular Neurobiology Hamburg University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dietmar","family":"Kuhl","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Cognition Center for Molecular Neurobiology Hamburg University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guido","family":"Hermey","sequence":"additional","affiliation":[{"name":"Institute for Molecular and Cellular Cognition Center for Molecular Neurobiology Hamburg University Medical Center Hamburg\u2010Eppendorf Hamburg 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